緊張性瞳孔で偶然発見されたRoss症候群の1例  [in Japanese] Ross syndrome presenting with asymptomatic tonic pupils  [in Japanese]

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Author(s)

    • 加藤 勇樹 Kato Yuki
    • 熊本大学大学院生命科学研究部神経内科学分野 Department of Neurology, Graduate School of Medical Sciences, Kumamoto University
    • 軸丸 美香 Jikumaru Mika
    • 熊本大学大学院生命科学研究部神経内科学分野 Department of Neurology, Graduate School of Medical Sciences, Kumamoto University
    • 大林 光念 Obayashi Konen
    • 熊本大学大学院生命科学研究部構造機能解析学分野 Department of Physiological and Morphological Sciences, Graduate School of Health Sciences, Kumamoto University
    • 安東 由喜雄 Ando Yukio
    • 熊本大学大学院生命科学研究部神経内科学分野 Department of Neurology, Graduate School of Medical Sciences, Kumamoto University

Abstract

66歳,女性.めまいを主訴に受診した際に,緊張性瞳孔と四肢腱反射消失をみとめた.また10歳代より,熱不耐症や左半身の発汗低下を自覚していた.ピロカルピン点眼試験では両側縮瞳,温熱発汗試験では顔面と左胸部に発汗低下をみとめ,Ross症候群と診断した.アセチルコリン皮内注射では鎖骨下と臍周囲の左側で発汗が低下していた.胃電図では平均周波数の低下とbradygastriaの頻度上昇,皮膚血流検査では左上肢で安静時皮膚血流増加と深呼吸刺激に対する血管反応性低下をみとめた.左上腕皮膚生検では毛包脂腺や汗腺は萎縮していた.免疫グロブリン静注療法は無効であった.本疾患では多様な自律神経障害が併存する可能性がある.

A 66-year-old woman presented with asymptomatic tonic pupils and areflexia when she was admitted to our hospital due to vertigo. She had also noticed heat intolerance and decreased sweating on her right side since she was approximately 10 years old. Both sides of each eye contracted in the pilocarpine eye drop test, while sweating on the face and left chest decreased. She was thus diagnosed with Ross syndrome. When acethylcholine was administered intracutaneously, sweating of the left subclavian and left peri-umbilical areas decreased. During an electrogastrogram, the average muscle contraction frequency was decreased and the incidence of bradygastria increased. Blood flow of the skin of the left forefinger (but not right) increased at rest and the reaction in the blood flow on deep breathing decreased. A skin biopsy of the left upper arm showed atrophy of pilosebaceous and sweat glands. Despite treatment with intravenous immunoglobulin, the patient's deficits did not improve. The lesions typical of Ross syndrome may be associated with many parts of the autonomic nervous system.

Journal

  • Rinsho Shinkeigaku

    Rinsho Shinkeigaku 55(3), 160-164, 2015

    Societas Neurologica Japonica

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